Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 9  |  Issue : 6  |  Page : 758-760  

Cystic hygroma: A surgical challenge


Department of Pediatric Surgery, VMMC and Safdarjung Hospital, New Delhi, India

Date of Web Publication16-Nov-2016

Correspondence Address:
Mohit Bhatia
1, Bank Colony, Opposite Old Sessions Courts, Ambala City - 134 003, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.194225

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  Abstract 

Cystic hygroma (CH) is a congenital malformation of lymphatic system. The incidence of CH is approximately 1 in 6000 live births. In most of the cases, it presents as a painless, fluctuant swelling. Due to its variable presentation its optimum treatment often poses a potential challenge.

Keywords: Cystic hygroma, lymphangioma, neck swelling


How to cite this article:
Bhatia M, Mattoo A. Cystic hygroma: A surgical challenge. Med J DY Patil Univ 2016;9:758-60

How to cite this URL:
Bhatia M, Mattoo A. Cystic hygroma: A surgical challenge. Med J DY Patil Univ [serial online] 2016 [cited 2024 Mar 28];9:758-60. Available from: https://journals.lww.com/mjdy/pages/default.aspx/text.asp?2016/9/6/758/194225


  Introduction Top


Cystic hygroma (CH) is a congenital entity which occurs due to failure in development of communication between lymphatic and venous system. The incidence of CH is approximately 1 in 6000 live births. [1] CH is considered as a developmental tumor of lymphatic origin and is a relatively rare lesion. [2] CH often presents as a swelling in head/neck region accounting for 70-80%, other sites includes mediastinum, axilla, pelvis. [3] Its treatment modalities comprise surgical excision, use of sclerosants, repeated aspiration, conservative management. We present a case of an 18-month-old male child with swelling around the left side of the face extending up to neck with a gradual progression and resulting in stridor and restricted mouth opening.


  Case Report Top


An 18-month-old male child weighing 8.8 kg presented to our hospital with swelling on the left side of face which had gradually increased with time for which patient was referred to a higher center. Swelling was minimal on the face at birth but had progressed considerably with time [Figure 1]. Initially injection bleomycin (intralesional) 0.5 mg/kg was administered. In spite of injection bleomycin, the lesion progressed further leading to stridor and restricted mouth opening. In course of time repeated aspiration was done under all aseptic conditions to improve patient symptomatically. Ultrasonography of neck region showed multilocular appearance with variable density with combination of fluid, soft tissue, and fat with cyst extending to anterior aspect of the neck. Magnetic resonance imaging (MRI) showed multiple cystic lesions within left masseter muscle and extending to anterior aspect of neck and into subcutaneous fat plane. Considering rapid increase in the size of the swelling and development of stridor patient was subjected for surgical procedure under general anesthesia [Figure 2]. Surgical enucleation was done, subplatysmal flaps were raised exposing the multicystic lesion [Figure 3]. Multiple cystic lesion was removed into preserving carotid vessels, jugular vessels, and spinal accessory nerve [Figure 4]. Histopathological findings were consistent with CH and patient was kept in Pediatric Intensive Care Unit for postoperative period. Postoperative period was uneventful.
Figure 1: Swelling extending from left side of face up to anterior aspect of neck

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Figure 2: Anticipated difficult intubation

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Figure 3: Multicystic mass being separated from surrounding tissue

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Figure 4: Important neurovascular structures preserved

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  Discussion Top


CH is lymphatic in origin which occurs due to sequestration or obstruction of lymphatic sacs. In more than 50% of cases, it presents at birth and in remaining cases it present by 2 years of age. However, in some extremely rare cases, CH has been reported in adulthood. [4]

CH is associated with various syndromes such as turner syndrome, Noonan syndrome, and fetal hydrops. [5] Around 70-80% of CH presents as a swelling in head/neck region however in some cases it can present at axilla, mediastinum, pelvis. [3] CH are classified as superficial CH (Lymphangioma circumscriptum) and deep ones (cavernous lymphangioma or cystic hygroma). [6]

Microscopically cyst wall consists of a single layer of flattened epithelium which may or may not contain vascular component, however, in some cases rapid enlargement/discoloration of the swelling can be attributed to the vascular component. [7] Characteristics of CH are fluctuant, brilliant transilluminant, and it never becomes malignant. [8] Diagnostic modalities to confirm the diagnosis of CH along with clinical suspicion includes ultrasonography (antenatal), computed tomography (CT) scan, MRI, which can give the exact extent of the lesion and regarding infiltration into adjoining structures. CT scan with contrast is better in localizing the lesion and its extent, but MRI is still considered better in localizing lesion due to its better accuracy and lack of artefacts. [9] Various treatment modalities have been considered for successful treatment of CH, which includes sclerosant agents, repeated aspiration, injection bleomycin, radiotherapy, surgical exploration. [10] Due to difficult and challenging surgical procedure owing to multicystic nature of the lesion with thin wall and proximity to vital neurovascular structures many nonsurgical options have been advocated which includes injection bleomycin administration intralesionally, a study by Oxfard et al. concluded that complete resolution had occurred in 44% cases and no response in 12% cases with use of bleomycin. [11] However, many authors have suggested the use of nonsurgical management in asymptomatic cases only, a study by Alqatein reported spontaneous regression is often associated with recurrence and hence is not valid treatment for CH and concluded that surgical excision of the lesion should be considered in all cases. [12]


  Conclusion Top


In developing countries like India where ignorance and lack of proper medical facilities at basic level prevails, cases of CH which are rare in occurrence and often report after considerable progression, therefore, their optimal treatment is delayed. Therefore, owing to the challenge posed by its variable and delayed presentation it becomes optimum to be rapid in diagnosing and treating a case of CH. Complete surgical excision should be considered treatment of choice in symptomatic cases, however, nonsurgical (conservative approach) can be tried initially for asymptomatic cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/ their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Dahnert W. Radiology Review Manual. 2 nd ed. Arizona: Williams and Wilkins; 1993.  Back to cited text no. 1
    
2.
Glasson MJ, Taylor SF. Cervical, cervicomediastinal and intrathoracic lymphangioma. Prog Pediatr Surg 1991;27:62-83.  Back to cited text no. 2
    
3.
Manikoth P, Mangalore GP, Megha V. Axillary cystic hygroma. J Postgrad Med 2004;50:215-6.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
4.
Naidu SI, McCalla MR. Lymphatic malformations of the head and neck in adults: A case report and review of the literature. Ann Otol Rhinol Laryngol 2004;113(3 Pt 1):218-22.  Back to cited text no. 4
    
5.
Osborne TE, Haller JA, Levin LS, Little BJ, King KE. Submandibular cystic hygroma resembling a plunging ranula in a neonate. Review and report of a case. Oral Surg Oral Med Oral Pathol 1991;71:16-20.  Back to cited text no. 5
    
6.
Mordehai J, Kurzbart E, Shinhar D, Sagi A, Finaly R, Mares AJ. Lymphangioma circumscriptum. Pediatr Surg Int 1998;13:208-10.  Back to cited text no. 6
    
7.
Shahriari A, Odell JA.Cervical and thoracic components of multiorgan lymphangiomatosis managed surgically. Ann Thorac Surg 2001;71:694-6.  Back to cited text no. 7
    
8.
Schloss MD, Sweet RC, Blais C, Tewfik TL. Lymphangioma in children. J Otolaryngol 1984;13:95-8.  Back to cited text no. 8
    
9.
Fisher R, Partington A, Dykes E. Cystic hygroma: Comparison between prenatal and postnatal diagnosis. J Pediatr Surg 1996;31:473-6.  Back to cited text no. 9
    
10.
Farmand M, Kuttenberger JJ. A new therapeutic concept for the treatment of cystic hygroma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1996;81:389-95.  Back to cited text no. 10
    
11.
Orford J, Barker A, Thonell S, King P, Murphy J. Bleomycin therapy for cystic hygroma. J Pediatr Surg 1995;30:1282-7.  Back to cited text no. 11
    
12.
Alqahtani A, Nguyen LT, Flageole H, Shaw K, Laberge JM. 25 years' experience with lymphangiomas in children. J Pediatr Surg 1999;34:1164-8.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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